Child Health/Sickle cell management

Sickle cell disease — drugs

also: penicillin prophylaxis · hydroxycarbamide · sickle crisis · hydroxyurea

Overview

Lifelong prophylaxis to prevent infection and crises, plus acute crisis management. The penicillin prophylaxis and hydroxycarbamide points are high-yield.

Mechanism

Hydroxycarbamide induces fetal haemoglobin (HbF), which interferes with HbS polymerisation → fewer vaso-occlusive crises and acute chest episodes. Penicillin prophylaxis + vaccination protect the functionally asplenic child from encapsulated organisms. Folic acid supports the high red-cell turnover.

Indications

Sickle cell disease (chronic prophylaxis)
Acute painful (vaso-occlusive) crisis

The agents

Penicillin V prophylaxisfrom 3 months

lifelong if splenic dysfunction

Plus pneumococcal/Hib/meningococcal vaccines (hyposplenism). Erythromycin if penicillin-allergic.

Hydroxycarbamidedisease-modifying

↑HbF → fewer crises/acute chest; for recurrent crises.

Folic acidsupplement

Chronic haemolysis.

Crisis: analgesia + fluids + O₂acute

opioids within 30 min

IV fluids, oxygen, warmth, treat infection; exchange transfusion for stroke/acute chest.

Adverse effects

Hydroxycarbamide: myelosuppressionserious

Monitor FBC.

Opioid dependence/tolerance in recurrent crisescommon

Avoid pethidine (seizures).

Transfusion iron overloadserious

Chelation if chronically transfused.

Cautions & contraindications

Hydroxycarbamide in pregnancypregnancy

Pethidine for crisis painall

Normeperidine → seizures.

Interactions

Hydroxycarbamide + other myelosuppressants

Monitoring & kinetics

FBC (hydroxycarbamide); vaccination status; growth,Crisis: analgesia within 30 min of arrival

Oral prophylaxis lifelong; acute crisis managed with analgesia/fluids/oxygen.

Choosing it

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Source: NICE — Sickle cell · BNF for Children — Hydroxycarbamide